Push yourself a little too hard at the gym and plan on waking up with sore muscles. Pull a muscle twisting the wrong way and feel pain sharp enough to affect your mobility. Muscle inflammation causes these common aches and pains, and normally the injury heals quickly without medical treatment. But what if the problem persists?
Emily Nicks loves to play softball. She played a lot of it as a 6-year-old in her hometown of Lemoore. She also competed with a dance team and as a cheerleader. The little athlete had certainly felt sore on occasion. But shortly after finishing first grade, Emily began to suffer from inexplicable, unrelenting muscle pain.
“In June 2006 we were at a cheerleading competition,” said Donnell Nicks, Emily’s mother. “We got word that a child had fallen. It was Emily.” Even as muscle weakness started interfering with her active lifestyle, a bothersome rash had already appeared on Emily’s thighs. Her doctor thought an allergic reaction had caused the symptoms, but after a trip to a Hanford allergist the issue remained unresolved.
Emily’s rash spread to other parts of her body and covered her face like a heart-shaped red stamp. By mid-July Emily was too weak to walk. “She was a good little softball player,” said Emily’s father, Ryan Nicks. “Every dad dreams of having an athlete and she was really good at softball.” But Emily’s illness changed all that. “I had to pick her up to put her on the sofa,” said Ryan. “I had to pick her up to put her in bed. I had to pick her up because she couldn’t do anything.”
Ryan and Donnell tried day after day to learn what had robbed their daughter of her vitality. All the while the severity of her symptoms dramatically increased. “It was like having an 80-pound infant,” said Donnell. “But she was alive and she was breathing, and that was all that mattered.”
A friend of Donnell’s recognized the symptoms plaguing Emily. “She said it was just like her mother,” said Ryan. Could Emily have the same rare disease? “I got online and looked it up,” he said. “When I read about the juvenile form, it was what Emily had to the ‘T.’ I thought this has got to be it. This must be what she has.”
Donnell praised their family practitioner for being open to exploring every possibility to learn the cause of Emily’s painful rash and sore, weakened muscles. The doctor agreed the answer might be found in a rare autoimmune disease known as Juvenile Dermatomyositis (JDM).
- dermato – skin
- myos – muscle
- -itis – inflammation
“On July 29, 2006 we got the call – take Emily to Children’s right away,” said Donnell.
Nearly two months passed between the onset of Emily’s symptoms and the diagnosis of JDM. “Two months felt like a year and a half,” said Ryan.
“She had a very serious disease,” said Dr. Dowain Wright, medical director of immunology and rheumatology at Children’s. Before current treatments for JDM were discovered, one-third of patients died and one-third became severely disabled. “Emily’s disease was particularly severe and required all known treatment,” he said. Treatment with potent medical therapies, including steroids and immune suppressants, has plunged the death rate to just 1 percent.
With his background in both pediatric immunology and pediatric rheumatology, Dr. Wright is well-suited to treat this rare autoimmune disease of the muscles and skin. “The incidence of new cases is two per year per million,” said Dr. Wright. “And that’s about right because we see at least two new patients with JDM each year.” The 10-county area of Central California served by Children’s Hospital includes nearly 1.2 million children under the age of 18. Emily and her parents would no longer have to fight this battle alone.
“We were so discouraged,” said Ryan. “But when we met Dr. Wright, he was like, ‘OK let’s go. Let’s kick this thing.’ We were so relieved. We were thinking we’d have to go way out of town.”
Donnell also expressed her gratitude for the doctor who saved their daughter’s life. “Dr. Wright was our savior,” she said.
When the compliment reached his ears, Dr. Wright waved it off and said, “It requires patience really, that’s all.”
“She was very sick when she came to us,” said Linda Miranda, registered nurse IV, rheumatology at Children's. Usually patients who experience a higher degree of severity in the muscles will present milder skin problems, and those with severe rashes will not suffer extreme muscle pain and weakness. Emily’s JDM attacked both her skin and muscles with such intensity that she spent her entire second-grade year bedridden.
“Months of seeing your kid wasting away…it was tough,” said Ryan. “My whole life changed. I used to be the strong-willed piece of iron.”
“You have to weigh the risks and the benefits when you’re treating a chronic illness,” said Miranda. “Dr. Wright is very aggressive in his approach to treating severe cases like Emily’s.”
“It’s a very long disease,” said Dr. Wright. “We tell families that up front. We tell them it will take at least three to five years of treatment to get the disease under control.” Dr. Wright’s aggressive treatment regimen began immediately in August 2006, and initially involved three days of IV infusions and one day of physical therapy per week, as well as daily oral medications.
Donnell explained her secret to giving a 6-year-old more than a dozen pills a day. “We crushed them in orange juice and put them in a shot glass,” said Donnell. She also employed creativity with their multiple excursions from Lemoore to Children’s Hospital. “We would make a special trip out of it,” she said. “I thought, ‘Let’s make the bad good.’”
“I try to block out all the bad and remember the good things,” said Ryan. “The little accomplishments, they were baby steps, but in my eyes it was like winning the Valley Championship.” Ryan is a football coach at Hanford High School, where the highest honor of the season goes to the team who wins the final game in the Valley playoffs. “I remember when Emily first picked up her head. It was huge!” Ryan exclaimed.
Dana Reed, pediatric physical therapist II at Children’s Hospital, was faced with the challenge of providing physical therapy to a child who could not walk, stand or sit unsupported. “We worked on things that would allow her to feel like she could do something on her own,” said Reed. “We did a lot of things lying down.”
“I’ll never forget when Donnell called from rehab and said, ‘Emily rolled over today.’” Ryan smiled at the memory. “That was such a big deal. She went from her stomach to her back. It doesn’t sound like much, but when you lie there lifeless, it’s a big deal.”
Donnell agreed. “Watching her progress was so awesome,” she said.
Emily connected with Reed, a former cheerleader for the 49ers. “We did some give and take,” said Reed. “For example, Emily likes cheerleading. We’d make up dances and cheers and she liked that. Then I made her do hard and scary things.”
“Dana wouldn’t let her quit,” said Ryan. “At first Emily probably hated her because she pushed her.” Emily grinned as she nodded to confirm her dad’s speculation.
“She was a little spitfire – which is good,” said Reed. “Crawling up and down a wedge mat was one of the hardest things she had to do. It required upper body and abdominal strength.” Reed remembers the tears of pain and fright little Emily expressed throughout the following year. “She had a lot of pain,” said Reed. “In March 2007 she had nine out of 10 on a pain intensity scale.”
Reed took Emily into the therapy pool when her skin irritations did not prevent it. Those sessions were among Emily’s favorites. “I felt free when I was in the water,” she said.
“I would try to make it as fun as possible,” said Reed. “But I didn’t want to waste any time. I didn’t want to waste her time or her parents’ time.” Reed’s work with Emily led to greater strength and endurance.
Dr Wright was pleased with Emily’s “myo” improvement, but the “dermato” issue persisted. Calcium-containing deposits formed in sheets along the muscles of Emily’s arms. The condition known as calcinosis is associated with inflamed or damaged tissues and affects an estimated 30 percent of children with untreated JDM. Emily’s severe skin rashes and diffuse calcifications made movement extremely painful and led to missed appointments with Reed. Emily continued her physical therapy until April 2008. “She was very fun to work with. Every time she came in was a new adventure,” said Reed.
Emily’s physical therapy sessions ended, but her trips to the Hospital’s ambulatory treatment center for IV infusions continue. Now in her sixth year of treatment for JDM, Emily’s visits are down to one full day per month. Ruth Cross, registered nurse III at Children’s Hospital, has watched Emily grow into a strong and beautiful 11-year-old. “Emily is very independent, driven and super sweet,” said Cross. “When Marcia, one of our main nurses, got sick, Emily pulled out a $10 bill to put in her card. She took it out of her pocket.” Cross’ voice broke and became softer. “Emily has been through a lot and Marcia was one of her favorite nurses,” she said to explain her emotion. “I told her, ‘You don’t have to do that,’ and she said she wanted to. It made me cry. Emily is a blessing.”
Dr. Wright calls Emily’s current condition “remission on medication,” which means the medication is controlling the disease. “Remission is defined as no disease activity in the skin or in the muscles,” he said. After five years of aggressive treatment, the “dermato” is finally showing the same improvement as the “myo.”
Emily’s battle against her childhood disease has been long and hard. “She would be in tears most of the time and now she’s happy,” said Cross. “She told me recently, ‘Thank you, I wouldn’t have been able to make it without you,’ and I didn’t understand at first. But now that I think back on how much pain she was in, I know what she meant by that.”
Dr. Wright’s success in forcing Emily’s rare disease into remission required patience and tenacity. Amazing people in the rheumatology clinic and the ambulatory treatment center continue to provide Emily and her family with incredible care. “To me this place is fabulous. I can’t express...” Ryan gave a grateful sigh. “There are no words to express how I feel about this Hospital.”
Now that Emily is back on her feet, she’s on a mission. “She’s full of all this energy that’s been stored up,” said Lynsey Silva, a family friend. “When she got well she was excited for everything – even going to a little pizza festival downtown. It was a big deal to her and Donnell because they couldn’t do that kind of thing for so long.”
“She played on the volleyball team this year!” exclaimed Donnell. “I didn’t miss a single game. I told my boss I’m taking off early on Fridays for awhile because I need to see my daughter play.”
In addition to enjoying sports again, Emily has returned to the stage. She raised a pig for 4H and was able to show it last summer. “It was her first time on stage since she got sick,” said Donnell, her throat thick with emotion. “I’d been so used to seeing her on stage, but that was a huge milestone.” More recently, Emily won a poetry reading contest at her school and qualified to participate in the Peach Blossom Festival at Fresno State. “She’s a performer,” said her proud mama. “She truly is a performer.”
“Emily’s a social butterfly, which sometimes makes it difficult for a teacher,” said Shelly Norris, Emily’s aunt and sixth-grade teacher. “But I understand that she missed a lot of interaction when she was sick.”
“She’s never gonna get those years back, but she’s definitely on her way to being a regular kid,” said Donnell.
“I have seen enormous growth in her physical abilities,” said Norris. “You wouldn’t even know that she had an illness.”
“She lost a lot, but just look at her,” said Ryan. “She’s doing really well now. I tell everyone at work she’s gonna do big things one day. She may even be the first female president of the United States.”
Once upon a time a beautiful swan thought he was an ugly duckling. The happy ending to the classic Hans Christian Andersen tale draws an exquisite picture of true beauty. Emily’s dad said she could write a book someday. She certainly has a story to tell. Perhaps Emily will write about doing the hard and scary things and keeping a sense of humor, or of losing so much and remaining generous, or of facing huge setbacks and emerging as a winner. Even if Emily’s story never finds its way to the pages of a book, it’s already written on the hearts of her friends and family. And it’s a real page turner.